What is the recommended management for childhood focal cerebral arteriopathy without ischemic stroke?

Management of Childhood Focal Cerebral Arteriopathy Without Ischemic Stroke

For children with focal cerebral arteriopathy who have not yet experienced an ischemic stroke, aspirin therapy at 3-5 mg/kg per day is reasonable for stroke prevention, particularly when there is ongoing cerebral artery stenosis, as no treatment may be associated with increased risk of stroke recurrence compared to any antithrombotic treatment. 1

Antiplatelet Therapy Approach

Aspirin remains the primary preventive strategy for children with focal cerebral arteriopathy:

• Initiate aspirin at 3-5 mg/kg per day as the standard dose for stroke prevention in children with documented arteriopathy 1
• If dose-related side effects occur (gastric distress or prolonged epistaxis), reduce to 1-3 mg/kg per day 1
• Continue treatment for a minimum of 3-5 years, or longer if cerebral artery stenosis persists on follow-up imaging 1

The rationale for aspirin therapy even without prior stroke is based on evidence that untreated children may have higher recurrence rates compared to those receiving any antithrombotic treatment, though the data comes from nonrandomized studies with inherent selection bias 1

Monitoring and Follow-Up Imaging

Serial vascular imaging is essential to differentiate focal cerebral arteriopathy from progressive arteriopathies:

• Focal cerebral arteriopathy typically shows nonprogressive unifocal and unilateral stenosis of the distal internal carotid artery or proximal middle/anterior cerebral arteries 2, 3
• Follow-up imaging demonstrates resolution or improvement in most cases, distinguishing it from moyamoya disease or other progressive vasculopathies 4, 2
• Children with more severe arteriopathy using the Focal Cerebral Arteriopathy Severity Score have larger strokes and higher risk of worsening, making imaging surveillance critical 3

Reye's Syndrome Prevention

Implement specific precautions to minimize Reye's syndrome risk in children on aspirin:

• Administer annual influenza vaccination 1
• Verify varicella vaccination status 1
• Withhold aspirin during confirmed or suspected influenza and varicella infections 1

There is no consensus on discontinuing aspirin during other febrile illnesses, though some experts recommend halving the dose during febrile illness while others favor continuation given the apparent increased stroke risk during such illnesses 1

Alternative Antiplatelet Agents

For children unable to tolerate aspirin:

• Clopidogrel at 1 mg/kg per day can be used as an alternative, though it may require compounding the 75 mg tablet for appropriate pediatric dosing 1
• Avoid dual antiplatelet therapy (aspirin plus clopidogrel) as this combination has been associated with subdural hemorrhage in children with arterial ischemic stroke, particularly those with diffuse vasculopathy and cerebral atrophy 1

Anticoagulation Considerations

Anticoagulation is generally reserved for specific high-risk scenarios rather than routine focal cerebral arteriopathy:

• Consider anticoagulation with warfarin (target INR 2.0-3.0) or LMWH for children with substantial risk of recurrent cardiac embolism, cervicocephalic arterial dissection, or selected hypercoagulable states 1
• Focal cerebral arteriopathy without these additional risk factors typically does not require anticoagulation 1

Clinical Context and Pitfalls

Key considerations specific to focal cerebral arteriopathy:

• Recent upper respiratory infection is strongly associated with focal cerebral arteriopathy, suggesting a possible inflammatory or infectious trigger 5
• The condition is most common in early school-age children (5-9 years) 5
• Approximately 20-25% of children with focal cerebral arteriopathy experience stroke recurrence, with concurrent infection being a risk factor for worsening 4, 3
• Diagnostic confusion is common—initial diagnoses of arterial dissection, moyamoya disease, or vasculitis are frequently revised to focal cerebral arteriopathy on follow-up imaging 4

Specialized Care Requirements

Children with focal cerebral arteriopathy should be managed at centers with:

• Pediatric stroke expertise and multidisciplinary teams 6
• Comprehensive neuroradiology services for serial vascular imaging 6, 7
• Access to neurosurgical consultation if arteriopathy progresses or stroke occurs 6

The favorable prognosis of focal cerebral arteriopathy (modified Rankin Scale 0-1 in most cases) supports conservative management with aspirin and close monitoring rather than aggressive interventions. 2, 3